title: The mitochondrial rhomboid protease: its rise from obscurity to the pinnacle of disease-relevant genes.
abstract: The Rhomboid proteases belong to a highly conserved family of proteins that are present in all branches of life. In Drosophila, the secretory pathway-localized rhomboid proteases are crucial for epidermal growth factor (EGF) signaling. The identification of a mitochondrial-localized rhomboid protease shed light on other functions of rhomboid proteases including the maintenance of mitochondrial morphology and the regulation of apoptosis. More recent work has revealed other functions of the mitochondrial rhomboid protease in mitochondrial and cellular biology, failure of which have been implicated in human diseases. In this review, we will summarize the current knowledge and disease relevance of the mitochondrial-localized rhomboid protease. This article is part of a Special Issue entitled: Intramembrane Proteases.
authors.affiliation: Department of Biochemistry, University of Toronto, Toronto, Ontario, Canada. Electronic address: eliana.chan@utoronto.ca.
authors.forename: Eliana Y L
authors.initials: EY
authors.lastname: Chan
authors.forename: G Angus
authors.initials: GA
authors.lastname: McQuibban
mesh_terms: D000818:Animals; D017209:Apoptosis; D017124:Conserved Sequence; D003924:Diabetes Mellitus, Type 2 / Q000201:enzymology* / Q000235:genetics / Q000473:pathology; D029721:Drosophila Proteins / Q000235:genetics / Q000378:metabolism; D004331:Drosophila melanogaster / Q000201:enzymology / Q000235:genetics; D005786:Gene Expression Regulation; D006801:Humans; D045726:Metalloproteases / Q000235:genetics / Q000378:metabolism*; D008928:Mitochondria / Q000201:enzymology* / Q000235:genetics; D024101:Mitochondrial Proteins / Q000235:genetics / Q000378:metabolism*; D010300:Parkinson Disease / Q000201:enzymology* / Q000235:genetics / Q000473:pathology; D059748:Proteolysis; D029701:Saccharomyces cerevisiae Proteins / Q000235:genetics / Q000378:metabolism; D015398:Signal Transduction; D013312:Stress, Physiological
chemical_list: D029721:Drosophila Proteins; D024101:Mitochondrial Proteins; D029701:Saccharomyces cerevisiae Proteins; C513147:rho-7 protein, Drosophila; D045726:Metalloproteases; C485223:PARL protein, human
keywords: Apoptosis; IMS; Intramembrane proteolysis; Mitochondrial fusion; Mitophagy; PD; Parkinson's disease; Substrate; T2D; TMD; intermembrane space; transmembrane domain; type 2 diabetes